Polycythemia vera is a chronic myeloproliferative disorder. Typically, the disease is characterized by panmyelosis, splenomegaly and a predisposition to venous/arterial thrombosis, myelofibrosis and acute leukemia. Principal tests used in the diagnosis of polycythemia vera include determining the red cell volume, the serum erythropoietin level, the arterial blood gas, the leukocyte alkaline phosphatase score, the serum vitamin B12 level, bone marrow biopsy, cytogenetics, and molecular markers. Further diagnostic means that can be employed are an abdominal computed tomography scan or a chest x-ray.
Phlebotomy alone or phlebotomy in combination with the administration of hydroxyurea or interferon currently constitute preferred approaches for treating polycythemia vera. However, newer, more effective drugs are needed to treat polycythemia vera in order to prevent progression of the illness to its inexorable end, severe fibrosis of the marrow (“marrow exhaustion”) or less commonly, acute leukemia.